Abstract
An epidemic illness characterized by head nodding associated with onchocerciasis has been described in eastern Africa since the early 1960s; we summarize published reports and recent studies. Onset of nodding occurs in previously healthy 5–15-year-old children and is often triggered by eating or cold temperatures and accompanied by cognitive impairment. Its incidence has increased in Uganda and South Sudan over the past 10 years. Four case–control studies identified modest and inconsistent associations. There were nonspecific lesions seen by magnetic resonance imaging, no cerebrospinal fluid inflammation, and markedly abnormal electroencephalography results. Nodding episodes are atonic seizures. Testing has failed to demonstrate associations with trypanosomiasis, cysticercosis, loiasis, lymphatic filariasis, cerebral malaria, measles, prion disease, or novel pathogens; or deficiencies of folate, cobalamin, pyridoxine, retinol, or zinc; or toxicity from mercury, copper, or homocysteine. There is a consistent enigmatic association with onchocerciasis detected by skin snip or serologic analysis. Nodding syndrome is an unexplained epidemic epilepsy.
Highlights
An epidemic illness characterized by head nodding associated with onchocerciasis has been described in eastern Africa since the early 1960s; we summarize published reports and recent studies
Nodding syndrome as a distinctive entity was reported from southern Sudan in the 1990s and investigated by local authorities and the World Health Organization (WHO) during 2001–2002 (1,2)
The term nodding disease was first applied in southern Sudan in the 1990s to describe the occurrence of repetitive head nodding, characteristically occurring among children while
Summary
Nodding syndrome as a distinctive entity was reported from southern Sudan in the 1990s and investigated by local authorities and the World Health Organization (WHO) during 2001–2002 (1,2). Thousands of cases have been reported from southern Tanzania, northern Uganda, and South Sudan, much smaller numbers have been documented and investigated in any detail (6–13). Detailed investigations that used consistently applied case definitions and active community outreach have identified >224 cases across Kitgum District in Uganda and 260 cases in Western Equatoria State in South Sudan; complete ascertainment of all cases was not the primary objective in either investigation (6,8,11). Anecdotal reports of deaths from drowning, burns, and other causes among nodding syndrome patients are common, but a collaborative effort between the Uganda Ministry of Health and CDC to register deaths and obtain autopsy specimens resulted in 1 autopsy over an 18-month period during 2011–2012. Community reports from a village administrator in Sudan (1) and focus groups in Uganda
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